Following exposure to an alkylating agent, haploinsufficiency of both
Egr1 and Apc promotes development of MDS; loss of
these genes together with Trp53 promotes AML development.
A, Kaplan–Meier survival curves of WT recipients transplanted with
Egr1+/−, Apcdel/+ BM cells
transduced with luc shRNA (EA-luc: black) or
Trp53 shRNA (EA-Trp53: red). Both donor and
recipient mice were treated with ENU. Disease development is significantly faster in
EA-Trp53 mice compared with EA-luc mice (200 days
vs. 370 days, P = 0.0014). B, Histologic classification
of diseases shows that most EA-luc mice developed MDS, and none
developed AML. C–F, Mouse genes were collapsed to human gene names;
differentially expressed genes in EA-Trp53 AML samples
(n = 6) versus EA-luc MDS samples
(n = 6) were analyzed using the GSEA software. Features, such as a
block in myeloid differentiation and upregulation of MYC target genes and
WNT/β-catenin signaling recapitulate human t-MN.